Background There has been great variation and uncertainty about how many and what CFTR mutations to include in cystic fibrosis (CF) newborn screening algorithms, and very little research on this topic using large populations of newborns. Methods We reviewed Wisconsin screening results for 1994–2008 to identify an ideal panel. Results Upon analyzing approximately 1 million screening results, we found it optimal to use a 23 CFTR mutation panel as a second tier when an immunoreactive trypsinogen (IRT)/DNA algorithm was applied for CF screening. This panel in association with a 96th percentile IRT cutoff gave a sensitivity of 97.3%, but restricting the DNA tier to F508del was associated with 90% (P<.0001). Conclusions Although CFTR panel selection has been challenging, our data show that a 23 mutation method optimizes sensitivity and is practically advantageous. The IRT cutoff value, however, is actually more critical than DNA in determining CF newborn screening sensitivity.

Intracardiac thrombus (ICT) is an intraoperative complication with high mortality that occurs during orthotopic liver transplantation (OLT). Patients with end‐stage liver disease have compromised coagulation pathways, and when combined with stressors of surgery, thrombi can form. However, it is unknown which patients are most likely to develop ICT. We performed a retrospective cohort study of all OLT patients at our hospital from 2010 to 2017 to identify risk factors for ICT. An analysis was performed with conventional bivariate tests and logistic regression. The incidence of ICT during OLT was 4.2% (22/528) with a 45.5% (10/22) mortality. Patients who developed ICT had higher physiologic Model for End‐Stage Liver Disease scores at the time of transplant (25.1 versus 32.4; P = 0.004), received grafts from donors with a higher body mass index (28.1 versus 32.2 kg/m2; P = 0.007), and had longer intraoperative warm ischemia times (53.1 versus 67.5 minutes; P = 0.001). The odds of developing ICT were significantly lower after administration of intravenous (IV) heparin prior to inferior vena cava (IVC) clamping compared with no administration of heparin (odds ratio, 0.25; 95% confidence interval, 0.08‐0.75; P = 0.01). In conclusion, the incidence of ICT at our institution is higher than previously reported, which may be explained by our routine use of transesophageal echocardiography. Although many factors associated with ICT in this study are nonmodifiable, administration of IV heparin prior to IVC cross‐clamping is modifiable and was found to be protective. Further studies will be needed to confirm findings and ultimately aid in preventing these lethal events.

Background With the rapid implementation of cystic fibrosis (CF) newborn screening (NBS), quality improvement (QI) has become essential to identify and prevent errors. Using Process Failure Modes and Effects Analysis (PFMEA), we adapted this method to determine if it could be applied to discover and rank high priority QI opportunities. Methods Site visits to three programmes were conducted, and PFMEA exercises were accomplished in Colorado, Massachusetts and Wisconsin with 23 experienced professionals. During each of these comprehensive sessions, participants identified and ranked potential failures based on severity, occurrence and detection to calculate risk priority number (RPN) values. Results A total of 96 failure modes were generated and ranked in a list of the 20 riskiest problems that show no significant discordances by site, although there were differences by profession of the rater, particularly nurses. Conclusions Our results illustrate that the PFMEA method applies well to CF NBS and that steps requiring communication and information transfer are perceived to be the highest risks. The number of identified failure makes and their potential impact demonstrate considerable overall risk and a need for ongoing QI.

The demand for liver transplants in the United States far exceeds the supply of organs. As need has increased, so has use of living donors. Coagulopathy and various side effects often preclude the use of neuraxial regional techniques and opioids for postoperative analgesia in patients with large “J” incisions. Here, we present a 25-year-old male undergoing a living donor hepatectomy who received quadratus lumborum catheters placed percutaneously after closure of incision and prior to emergence to provide excellent analgesia and a viable opioid-sparing approach. Quadratus lumborum catheters are a safe option for a multimodal, opioid-sparing approach to analgesia.

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